Division of Hematology/Oncology, Seattle Children's, Seattle, Washington, USA.
Cancer. 2011 Jul 15;117(14):3262-7. doi: 10.1002/cncr.25856. Epub 2011 Jan 18.
Contemporary therapy for medulloblastoma results in adverse neurocognitive effects on young children, particularly those under the age of 3. Stratification of patients by risk group may allow toxic treatment to be avoided.
Seventy-six patients diagnosed with medulloblastoma and enrolled on CCG-9921 underwent central review of pathology, and histologic subtype was designated as desmoplastic or nondesmoplastic. Nonparametric event-free survival (EFS) and survival (OS) curves were computed using the product limit (Kaplan-Meier) estimates, and the log-rank test was used to compare survival according to histologic subtype.
Patients with desmoplastic medulloblastoma experienced a favorable EFS of 77% ± 9% and OS of 85% ± 8% compared with EFS of 17% ± 5% and OS of 29% ± 6% for patients with tumors in the nondesmoplastic group (P < .0001 for both EFS and OS comparisons). Patients without disease progression did not receive radiation therapy.
Children less than 3 with desmoplastic histology of medulloblastoma represent a lower-risk group for whom reduction of therapy, including elimination of radiation therapy, is an appropriate strategy.
当代治疗髓母细胞瘤的方法会对幼儿，尤其是 3 岁以下的幼儿造成不良的神经认知影响。通过风险组分层，可以避免对患者进行有毒治疗。
76 名被诊断为髓母细胞瘤并参与 CCG-9921 研究的患者接受了中央病理复查，组织学亚型被指定为促纤维增生型或非促纤维增生型。使用乘积限（Kaplan-Meier）估计来计算无事件生存（EFS）和总生存（OS）曲线，对数秩检验用于根据组织学亚型比较生存情况。
与非促纤维增生型组的 EFS 为 17%±5%和 OS 为 29%±6%相比，促纤维增生型组的 EFS 为 77%±9%和 OS 为 85%±8%，促纤维增生型组患者的 EFS 和 OS 均显著更高（P<0.0001）。未发生疾病进展的患者未接受放射治疗。
3 岁以下具有促纤维增生型组织学特征的髓母细胞瘤患儿是低风险组，减少治疗，包括消除放射治疗，是一种合理的策略。
